atrophic muscular disorders

Summary

Summary: Disorders characterized by an abnormal reduction in muscle volume due to a decrease in the size or number of muscle fibers. Atrophy may result from diseases intrinsic to muscle tissue (e.g., MUSCULAR DYSTROPHY) or secondary to PERIPHERAL NERVOUS SYSTEM DISEASES that impair innervation to muscle tissue (e.g., MUSCULAR ATROPHY, SPINAL).

Top Publications

  1. Jackman R, Kandarian S. The molecular basis of skeletal muscle atrophy. Am J Physiol Cell Physiol. 2004;287:C834-43 pubmed
    ..We also discuss how ongoing work should uncover more about the molecular underpinnings of muscle wasting, particularly that due to disuse...
  2. Adachi H, Kume A, Li M, Nakagomi Y, Niwa H, Do J, et al. Transgenic mice with an expanded CAG repeat controlled by the human AR promoter show polyglutamine nuclear inclusions and neuronal dysfunction without neuronal cell death. Hum Mol Genet. 2001;10:1039-48 pubmed
  3. Pomatto E, Castellano S, Bianchi S. Unilateral fibroadipose degeneration of the masticatory muscles. Dentomaxillofac Radiol. 2001;30:346-8 pubmed
    ..Myotomy of masseter and internal pterygoid muscles and coronoidotomy improved his symptoms. There is no known cause of fibroadipose replacement of muscle fibres...
  4. Symons T, Sheffield Moore M, Chinkes D, Ferrando A, Paddon Jones D. Artificial gravity maintains skeletal muscle protein synthesis during 21 days of simulated microgravity. J Appl Physiol (1985). 2009;107:34-8 pubmed publisher
  5. Chen C, Chen P, Yao M, Chu J, Chan W. MRI of hemangioblastoma in the conus medullaris. Comput Med Imaging Graph. 2008;32:78-81 pubmed
    ..Histological findings were compatible with the diagnosis of hemangioblastoma. Hemangioblastoma should be included in the differential diagnosis in patients with an enhancing tumor and adjacent engorged vessels of the spinal cord...
  6. Pandey U, Nie Z, Batlevi Y, McCray B, Ritson G, Nedelsky N, et al. HDAC6 rescues neurodegeneration and provides an essential link between autophagy and the UPS. Nature. 2007;447:859-63 pubmed
    ..Morover, these findings suggest that it may be possible to intervene in neurodegeneration by augmenting HDAC6 to enhance autophagy...
  7. Coutinho E, Gomes A, Franca C, Salvini T. A new model for the immobilization of the rat hind limb. Braz J Med Biol Res. 2002;35:1329-32 pubmed
    ..Immobilization was effective in producing chronic muscle disuse in the hind limbs of rats and is an acceptable alternative to the traditional methods of immobilization such as cast or plaster cast...
  8. Sasa T, Sairyo K, Yoshida N, Fukunaga M, Koga K, Ishikawa M, et al. Continuous muscle stretch prevents disuse muscle atrophy and deterioration of its oxidative capacity in rat tail-suspension models. Am J Phys Med Rehabil. 2004;83:851-6 pubmed
    ..The purpose of this study was to evaluate the effect of continuous muscle stretch on disuse-atrophied muscles...
  9. Poletti A. The polyglutamine tract of androgen receptor: from functions to dysfunctions in motor neurons. Front Neuroendocrinol. 2004;25:1-26 pubmed
    ..This review summarizes the mechanisms of neurotoxicity associated to androgen receptor containing an elongated polyglutamine tract responsible for motor neuronal cell death...

More Information

Publications62

  1. Waza M, Adachi H, Katsuno M, Minamiyama M, Tanaka F, Doyu M, et al. Modulation of Hsp90 function in neurodegenerative disorders: a molecular-targeted therapy against disease-causing protein. J Mol Med (Berl). 2006;84:635-46 pubmed
    ..This review will consider our research findings and discuss the possibility of a clinical application of 17-AAG to SBMA and other neurodegenerative diseases...
  2. Wu X, Baer L, Wolf S, Wade C, Walters T. The impact of muscle disuse on muscle atrophy in severely burned rats. J Surg Res. 2010;164:e243-51 pubmed publisher
    ..In this study, we sought to determine whether muscle disuse has additional impact on muscle atrophy after severe burn using a rat model combining severe cutaneous burn and hindlimb unloading...
  3. Banno H, Katsuno M, Suzuki K, Iguchi Y, Adachi H, Tanaka F, et al. [Molecular-targeted therapy for motor neuron disease]. Brain Nerve. 2009;61:891-900 pubmed
    ..Oral administration of sodium butyrate--a histone deacetylase inhibitor--resulted in the improvement of neurological dysfunction in the SBMA mouse model, although its therapeutic dose range is narrow...
  4. Tomik B, Partyka D, Sułek A, Kurek Gryz E, Banach M, Ostrowska M, et al. A phenotypic-genetic study of a group of Polish patients with spinal and bulbar muscular atrophy. Amyotroph Lateral Scler. 2006;7:72-9 pubmed
    ..The extended CAG repeats within families were stable...
  5. Pandit L, Agrawal A. Neuromuscular disorders in critical illness. Clin Neurol Neurosurg. 2006;108:621-7 pubmed
    ..This update focuses on neuromuscular disorders occurring in the critical care set up associated with diffuse and severe weakness...
  6. Piccioni F, Roman B, Fischbeck K, Taylor J. A screen for drugs that protect against the cytotoxicity of polyglutamine-expanded androgen receptor. Hum Mol Genet. 2004;13:437-46 pubmed
    ..The fourth compound, suloctidil, is a calcium channel blocker...
  7. Alzghoul M, Gerrard D, Watkins B, Hannon K. Ectopic expression of IGF-I and Shh by skeletal muscle inhibits disuse-mediated skeletal muscle atrophy and bone osteopenia in vivo. FASEB J. 2004;18:221-3 pubmed
    ..These results support the theory that skeletal muscle can regulate bone maintenance and could offer potentially novel and efficient therapeutic options for attenuating muscle and bone atrophy during aging, illness and spaceflight...
  8. Lund A, Udd B, Juvonen V, Andersen P, Cederquist K, Ronnevi L, et al. Founder effect in spinal and bulbar muscular atrophy (SBMA) in Scandinavia. Eur J Hum Genet. 2000;8:631-6 pubmed
    ..The data suggest that the SBMA mutation was introduced into western Finland 20 generations ago. Haplotype analysis implies a common ancestor for the majority of Scandinavian SBMA patients...
  9. Endo T. Stem cells and plasticity of skeletal muscle cell differentiation: potential application to cell therapy for degenerative muscular diseases. Regen Med. 2007;2:243-56 pubmed
    ..In addition, fibroblasts or undifferentiated myoblasts treated with a drug acquire multipotency. These cells may open new doors in cell therapy...
  10. Muller F, Dehail P, Bestaven E, Petit J, Joseph P, Barat M, et al. Maximal and sustained isokinetic lower-limb muscle strength in hospitalized older people. Muscle Nerve. 2007;35:739-44 pubmed
    ..It could be related to the modifications of muscle-fiber composition described in elderly subjects and enhanced by hospitalization...
  11. Puget M, Iwaz J, Tristan A, Streichenberger N. Whipple's disease with muscle impairment. Muscle Nerve. 2006;34:794-8 pubmed
    ..Ceftriaxone led to clinical improvement. Although rare, Whipple's disease should be considered in the differential diagnosis of diffuse myopathy...
  12. Fanghanel J, Proff P, Dietze S, Bayerlein T, Mack F, Gedrange T. The morphological and clinical relevance of mandibular and maxillary bone structures for implantation. Folia Morphol (Warsz). 2006;65:49-53 pubmed
    ..Osseointegration is performed at an early stage (which includes bleeding, granulation tissue, foreign-body recognition, interactions) and at a late stage (so-called osseous bridging, development of fibrous and lamellar bone)...
  13. Heeschen C, Deu A, Langenbrink L, Goldmann B, Hamm C. Analytical and diagnostic performance of troponin assays in patients suspicious for acute coronary syndromes. Clin Biochem. 2000;33:359-68 pubmed
    ..The controversy whether there is a clinically significant difference between troponin T (cTnT) and troponin I (cTnI) in regard to predictive value and cardiac specificity is still ongoing...
  14. Hanajima R, Terao Y, Nakatani Enomoto S, Hamada M, Yugeta A, Matsumoto H, et al. Postural tremor in X-linked spinal and bulbar muscular atrophy. Mov Disord. 2009;24:2063-9 pubmed publisher
    ..Subclinical sensory disturbance or a decrease of motor unit numbers might be candidates for such peripheral factors contributing to tremor genesis in SBMA...
  15. Bortz W. Disuse and aging, 2009. J Gerontol A Biol Sci Med Sci. 2010;65:382-5 pubmed publisher
    ..Knowledge of the basic processes determinative of the life courses of older persons has progressed dramatically in the past few years. This article is an update of a similar survey performed 27 years ago...
  16. Nedelsky N, Pennuto M, Smith R, Palazzolo I, Moore J, Nie Z, et al. Native functions of the androgen receptor are essential to pathogenesis in a Drosophila model of spinobulbar muscular atrophy. Neuron. 2010;67:936-52 pubmed publisher
    ..These findings indicate that SBMA pathogenesis is mediated by misappropriation of native protein function, a mechanism that may apply broadly to polyglutamine diseases...
  17. Shimohata T, Kimura T, Nishizawa M, Onodera O, Tsuji S. Five year follow up of a patient with spinal and bulbar muscular atrophy treated with leuprorelin. J Neurol Neurosurg Psychiatry. 2004;75:1206-7 pubmed
  18. Gross T, Poliachik S, Prasad J, Bain S. The effect of muscle dysfunction on bone mass and morphology. J Musculoskelet Neuronal Interact. 2010;10:25-34 pubmed
    ..The specific signaling pathways by which this interdependency is achieved, however, remain to be clarified...
  19. Ibi T, Ohno K, Sahashi K, Nakao N, Kondo H. Progressive myopathy with circulating autoantibody against giantin in the Golgi apparatus. Neurology. 2004;62:1891-3 pubmed
  20. Mishra A, Dikshit P, Purkayastha S, Sharma J, Nukina N, Jana N. E6-AP promotes misfolded polyglutamine proteins for proteasomal degradation and suppresses polyglutamine protein aggregation and toxicity. J Biol Chem. 2008;283:7648-56 pubmed publisher
    ..These findings suggest that E6-AP is a critical mediator of the neuronal response to misfolded polyglutamine proteins and represents a potential therapeutic target in the polyglutamine diseases...
  21. de Boer M, Selby A, Atherton P, Smith K, Seynnes O, Maganaris C, et al. The temporal responses of protein synthesis, gene expression and cell signalling in human quadriceps muscle and patellar tendon to disuse. J Physiol. 2007;585:241-51 pubmed
  22. Murata T, Suzuki E, Ito S, Sawatsubashi S, Zhao Y, Yamagata K, et al. RNA-binding protein hoip accelerates polyQ-induced neurodegeneration in Drosophila. Biosci Biotechnol Biochem. 2008;72:2255-61 pubmed
    ..These findings suggest that the process of neurodegeneration induced by abnormal polyQ expansion in the hAR may be regulated by the activity of snoRNP complex...
  23. Abresch R, Carter G, Jensen M, Kilmer D. Assessment of pain and health-related quality of life in slowly progressive neuromuscular disease. Am J Hosp Palliat Care. 2002;19:39-48 pubmed
    ..In conclusion, with the exception of adult SMA, the frequency and severity of pain reported in slowly progressive NMDs was significant...
  24. McDonagh J, Callister R, Favron M, Stuart D. Resistance to disuse atrophy in a turtle hindlimb muscle. J Comp Physiol A Neuroethol Sens Neural Behav Physiol. 2004;190:321-9 pubmed
    ..It is suggested that such resistance to disuse is due to intrinsic mechanisms that enable heterothermic mammals and ectothermic vertebrates to tolerate an unfavorable climate and food and water shortages by using hypometabolic states...
  25. Mallinson J, Constantin Teodosiu D, Sidaway J, Westwood F, Greenhaff P. Blunted Akt/FOXO signalling and activation of genes controlling atrophy and fuel use in statin myopathy. J Physiol. 2009;587:219-30 pubmed publisher
    ..These changes occurred in the main before evidence of extensive myopathy or a decline in the muscle protein to DNA ratio...
  26. Roy R, Zhong H, Khalili N, Kim S, Higuchi N, Monti R, et al. Is spinal cord isolation a good model of muscle disuse?. Muscle Nerve. 2007;35:312-21 pubmed
    ..These results indicate that SI eliminates nearly all of the normal EMG activity in the hindlimb muscles in the presence of relatively normal muscle innervation and functional intraspinal neural circuitry...
  27. Marzetti E, Hwang J, Lees H, Wohlgemuth S, Dupont Versteegden E, Carter C, et al. Mitochondrial death effectors: relevance to sarcopenia and disuse muscle atrophy. Biochim Biophys Acta. 2010;1800:235-44 pubmed publisher
    ..Furthermore, a translational effort is required to determine the role played by apoptosis in the pathogenesis of sarcopenia and disuse-induced muscle loss in human subjects...
  28. Tarlac V, Storey E. Role of proteolysis in polyglutamine disorders. J Neurosci Res. 2003;74:406-16 pubmed
    ..Inhibition of specific proteases constitutes a potential therapeutic approach in these diseases...
  29. Lentle R, Kruger M. Changes in mineralization and biomechanics of tibial metaphyses in splinted rats. J Appl Physiol (1985). 2005;99:173-80 pubmed
    ..Thus osteoporotic changes consequent on immobilization include both local effects on mineralization and general effects on growth, which may separately influence the elastic properties of bone...
  30. Arai H, Tanabe Y, Hachiya Y, Otsuka E, Kumada S, Furushima W, et al. Finger cold-induced vasodilatation, sympathetic skin response, and R-R interval variation in patients with progressive spinal muscular atrophy. J Child Neurol. 2005;20:871-5 pubmed
    ..Results show that some patients with spinal muscular atrophy have autonomic dysfunction, especially sympathetic nerve hyperactivity, that resembles dysfunction observed in amyotrophic lateral sclerosis...
  31. Walsh F, Celeste A. Myostatin: a modulator of skeletal-muscle stem cells. Biochem Soc Trans. 2005;33:1513-7 pubmed
    ..These results validate myostatin inhibition as a therapeutic approach to muscle wasting diseases such as muscular dystrophy, sarcopenic frailty of the elderly and amylotrophic lateral sclerosis...
  32. Pearce J. Dystrophy, abiotrophy, amyotrophy. Eur Neurol. 2005;54:54 pubmed
  33. Katsuno M, Adachi H, Minamiyama M, Waza M, Doi H, Kondo N, et al. Disrupted transforming growth factor-beta signaling in spinal and bulbar muscular atrophy. J Neurosci. 2010;30:5702-12 pubmed publisher
    ..The present study thus indicates that disruption of TGF-beta due to the transcriptional dysregulation of TbetaRII is associated with polyglutamine-induced motor neuron damage in SBMA...
  34. Tanaka F, Sobue G. [Integrated molecular medicine for neuronal and neoplastic disorders]. Seikagaku. 2007;79:121-30 pubmed
  35. Desaphy J, Pierno S, Liantonio A, Giannuzzi V, Digennaro C, Dinardo M, et al. Antioxidant treatment of hindlimb-unloaded mouse counteracts fiber type transition but not atrophy of disused muscles. Pharmacol Res. 2010;61:553-63 pubmed publisher
    ..Trolox treatment had no effect on control mice. These results suggest possible benefits of antioxidants in protecting muscle against disuse...
  36. Davies D, Irwin P. Degenerative neurological and neuromuscular disease in young rottweilers. J Small Anim Pract. 2003;44:388-94 pubmed
    ..Knowledge of these syndromes and a methodical approach to neurological diagnosis can help the veterinarian to identify the underlying disease and establish a prognosis when presented with a tetraparetic or ataxic young rottweiler...
  37. Jones S, Hill R, Krasney P, O Conner B, Peirce N, Greenhaff P. Disuse atrophy and exercise rehabilitation in humans profoundly affects the expression of genes associated with the regulation of skeletal muscle mass. FASEB J. 2004;18:1025-7 pubmed
  38. Sułek A, Hoffman Zacharska D, Krysa W, Szirkowiec W, Fidziańska E, Zaremba J. CAG repeat polymorphism in the androgen receptor (AR) gene of SBMA patients and a control group. J Appl Genet. 2005;46:237-9 pubmed
    ..Normal and abnormal ranges of CAG repeats were established in the control group and in 21 patients whose clinical diagnosis of SBMA was molecularly confirmed. The ranges are similar to those reported for other populations...
  39. Beitel L, Scanlon T, Gottlieb B, Trifiro M. Progress in Spinobulbar muscular atrophy research: insights into neuronal dysfunction caused by the polyglutamine-expanded androgen receptor. Neurotox Res. 2005;7:219-30 pubmed
  40. Vargas R, Lang C. Alcohol accelerates loss of muscle and impairs recovery of muscle mass resulting from disuse atrophy. Alcohol Clin Exp Res. 2008;32:128-37 pubmed
    ..Therefore, the present study addressed the hypothesis that EtOH accelerates the disuse atrophy and/or impairs the accretion of muscle protein during muscle recovery...
  41. Evert B, Wullner U, Klockgether T. Cell death in polyglutamine diseases. Cell Tissue Res. 2000;301:189-204 pubmed
  42. Hinkle R, Donnelly E, Cody D, Samuelsson S, Lange J, Bauer M, et al. Activation of the CRF 2 receptor modulates skeletal muscle mass under physiological and pathological conditions. Am J Physiol Endocrinol Metab. 2003;285:E889-98 pubmed
    ..In addition, selective activation of the CRF2R increased nonatrophy skeletal muscle mass. Thus we describe for the first time a novel activity of the CRF2R, modulation of skeletal muscle mass...
  43. O CONNELL K, Gannon J, Doran P, Ohlendieck K. Proteomic profiling reveals a severely perturbed protein expression pattern in aged skeletal muscle. Int J Mol Med. 2007;20:145-53 pubmed
  44. Tam S, Archibald V, Tyreman N, Gordon T. Effect of exercise on stability of chronically enlarged motor units. Muscle Nerve. 2002;25:359-69 pubmed
  45. Leeuw M, Goossens M, Linton S, Crombez G, Boersma K, Vlaeyen J. The fear-avoidance model of musculoskeletal pain: current state of scientific evidence. J Behav Med. 2007;30:77-94 pubmed
    ..Finally, available evidence on recent clinical applications is provided, and unresolved issues that need further exploration are discussed...
  46. Dodd S, Gagnon B, Senf S, Hain B, Judge A. Ros-mediated activation of NF-kappaB and Foxo during muscle disuse. Muscle Nerve. 2010;41:110-3 pubmed publisher
    ..Thus, H2O2 may be an important initiator of these signaling pathways that lead to muscle atrophy. ..
  47. Brocca L, Pellegrino M, Desaphy J, Pierno S, Camerino D, Bottinelli R. Is oxidative stress a cause or consequence of disuse muscle atrophy in mice? A proteomic approach in hindlimb-unloaded mice. Exp Physiol. 2010;95:331-50 pubmed publisher
    ..Trolox administration did not prevent metabolic adaptations in either Sol or Gas. The present findings suggest that oxidative stress is not a major determinant of muscle atrophy in HU mice...
  48. Serratrice G. Clinical semiology of neuromuscular diseases (hand muscles). Acta Myol. 2005;24:44-6 pubmed
  49. Kubota A, Sakuraba K, Sawaki K, Sumide T, Tamura Y. Prevention of disuse muscular weakness by restriction of blood flow. Med Sci Sports Exerc. 2008;40:529-34 pubmed publisher
    ..The aim of the present study was to compare the effects of periodic restriction of blood flow to lower extremities with those of isometric exercise on disuse muscular atrophy and weakness induced by immobilization and unloading...
  50. Hanson A, Ferguson V, Simske S, Cannon C, Stodieck L. Comparison of tail-suspension and sciatic nerve crush on the musculoskeletal system in young-adult mice. Biomed Sci Instrum. 2005;41:92-6 pubmed
    ..These unloading models have tissue-specific effects that impact their applications for musculoskeletal research...
  51. Minamiyama M, Katsuno M, Adachi H, Waza M, Sang C, Kobayashi Y, et al. Sodium butyrate ameliorates phenotypic expression in a transgenic mouse model of spinal and bulbar muscular atrophy. Hum Mol Genet. 2004;13:1183-92 pubmed
    ..Our results indicate that SB is a possible therapeutic agent for SBMA and other polyQ diseases, although an appropriate dose should be determined for clinical application...
  52. Bonetto A, Penna F, Muscaritoli M, Minero V, Rossi Fanelli F, Baccino F, et al. Are antioxidants useful for treating skeletal muscle atrophy?. Free Radic Biol Med. 2009;47:906-16 pubmed publisher
    ..This review focuses on the possibility of using antioxidant treatments to target molecular pathways involved in the pathogenesis of skeletal muscle wasting...
  53. Pierno S, Desaphy J, Liantonio A, De Bellis M, Bianco G, De Luca A, et al. Change of chloride ion channel conductance is an early event of slow-to-fast fibre type transition during unloading-induced muscle disuse. Brain. 2002;125:1510-21 pubmed
    ..Pharmacological modulation of ClC-1 channels may help to prevent disuse-induced muscle impairment...